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Oxidative stress in SEPN1-related myopathy: From pathophysiology to treatment
Annals of Neurology , 06/30/09
Arbogast S et al. - SelN plays a key role in redox homeostasis and human cell protection against oxidative stress. Oxidative/nitrosative stress is a primary pathogenic mechanism in SEPN1-RM, which can be effectively targeted ex vivo by antioxidants. These findings pave the way to SEPN1-RM treatment, which would represent a first specific pharmacological treatment for a congenital myopathy.
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